Exocrine pancreatic neoplasms represent up to 95% of pancreatic cancers (PCs) and are widely recognized among the most lethal solid cancers, with a very poor 5-year survival rate of 5%-10%. The remaining < 5% of PCs are neuroendocrine tumors that are usually characterized by a better prognosis, with a median overall survival of 3.6 years. The most common type of PC is pancreatic ductal adenocarcinoma (PDAC), which accounts for roughly 85% of all exocrine PCs. However up to 10% of exocrine PCs have rare histotypes, which are still poorly understood. These subtypes can be distinguished from PDAC in terms of pathology, imaging, clinical presentation and prognosis. Additionally, due to their rarity, any knowledge regarding these specific histotypes is mostly based on case reports and a small series of retrospective analyses. Therefore, treatment strategies are generally deduced from those used for PDAC, even if these patients are often excluded or not clearly represented in clinical trials for PDAC. For these reasons, it is essential to collect as much information as possible on the management of PC, as assimilating it with PDAC may lead to the potential mistreatment of these patients. Here, we report the most significant literature regarding the epidemiology, typical presentation, possible treatment strategies, and prognosis of the most relevant histotypes among rare PCs.

One size does not fit all for pancreatic cancers : a review on rare histologies and therapeutic approaches / M. Niger, M. Prisciandaro, M. Antista, M.A.T. Monica, L. Cattaneo, N. Prinzi, S. Manglaviti, F. Nichetti, M. Brambilla, M. Torchio, F. Corti, S. Pusceddu, J. Coppa, V. Mazzaferro, F. de Braud, M. Di Bartolomeo. - In: WORLD JOURNAL OF GASTROINTESTINAL ONCOLOGY. - ISSN 1948-5204. - 12:8(2020 Aug 15), pp. 833-849.

One size does not fit all for pancreatic cancers : a review on rare histologies and therapeutic approaches

V. Mazzaferro;F. de Braud;
2020

Abstract

Exocrine pancreatic neoplasms represent up to 95% of pancreatic cancers (PCs) and are widely recognized among the most lethal solid cancers, with a very poor 5-year survival rate of 5%-10%. The remaining < 5% of PCs are neuroendocrine tumors that are usually characterized by a better prognosis, with a median overall survival of 3.6 years. The most common type of PC is pancreatic ductal adenocarcinoma (PDAC), which accounts for roughly 85% of all exocrine PCs. However up to 10% of exocrine PCs have rare histotypes, which are still poorly understood. These subtypes can be distinguished from PDAC in terms of pathology, imaging, clinical presentation and prognosis. Additionally, due to their rarity, any knowledge regarding these specific histotypes is mostly based on case reports and a small series of retrospective analyses. Therefore, treatment strategies are generally deduced from those used for PDAC, even if these patients are often excluded or not clearly represented in clinical trials for PDAC. For these reasons, it is essential to collect as much information as possible on the management of PC, as assimilating it with PDAC may lead to the potential mistreatment of these patients. Here, we report the most significant literature regarding the epidemiology, typical presentation, possible treatment strategies, and prognosis of the most relevant histotypes among rare PCs.
Pancreatic acinar cell cancer; Pancreatic adenosquamous cancer; Pancreatoblastoma; Pseudopapillary pancreatic cancer; Rare pancreatic cancers; Undifferentiated pancreatic cancer; Pancreatic acinar cell cancer; Pancreatic adenosquamous cancer; Pancreatoblastoma; Pseudopapillary pancreatic cancer; Rare pancreatic cancers; Undifferentiated pancreatic cancer
Settore MED/06 - Oncologia Medica
Settore MED/18 - Chirurgia Generale
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/2434/839877
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